J Korean Med Sci.  2012 Nov;27(11):1378-1384. 10.3346/jkms.2012.27.11.1378.

Successful Treatment of Primary Central Nervous System Lymphoma without Irradiation in Children: Single Center Experience

Affiliations
  • 1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. hyshin@snu.ac.kr
  • 2Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
  • 3Center for Pediatric Oncology, National Cancer Center, Goyang, Korea.

Abstract

Primary CNS lymphoma (PCNSL) is a very uncommon disease in children, and usually treated by chemotherapy, combined with focal or craniospinal radiotherapy (RT). However, adverse effects of RT are a concern. We evaluated the outcomes of childhood PCNSL, treated with systemic and intrathecal chemotherapy, but without RT. For fifteen years, six patients among 175 of non-Hodgkin lymphoma were diagnosed as PCNSL in Seoul National University Children's Hospital and we analyzed their medical records retrospectively. Their male:female ratio was 5:1, and median age was 10.1 yr. The primary sites were the sellar area in three patients, parietal area in one, cerebellum in one, and multiple areas in one. Their pathologic diagnoses were diffuse large B-cell lymphoma in three patients, Burkitt lymphoma in two, and undifferentiated B-cell lymphoma in one. Five were treated with the LMB96 treatment protocol, and one was treated with the CCG-106B protocol. None had RT as a first-line treatment. One patient had a local relapse and received RT and salvage chemotherapy, without success. No patient had treatment-related mortality. Their estimated 5-yr event-free and overall survival rates were both 83.3%. In conclusion, PCNSL is a rare disease in childhood, but successfully treated by chemotherapy without RT.

Keyword

Primary CNS Lymphoma; Children; Irradiation

MeSH Terms

Adolescent
Antineoplastic Combined Chemotherapy Protocols/*therapeutic use
Central Nervous System Neoplasms/diagnosis/*drug therapy
Child
Child, Preschool
Cyclophosphamide/therapeutic use
Cytarabine/therapeutic use
Disease-Free Survival
Doxorubicin/therapeutic use
Etoposide/therapeutic use
Female
Humans
Hydrocortisone/therapeutic use
Infant
Leucovorin/therapeutic use
Lymphoma, Non-Hodgkin/diagnosis/*drug therapy
Male
Methotrexate/therapeutic use
Prednisone/therapeutic use
Recurrence
Retrospective Studies
Tomography, X-Ray Computed
Treatment Outcome
Vincristine/therapeutic use
Cytarabine
Doxorubicin
Etoposide
Cyclophosphamide
Hydrocortisone
Prednisone
Vincristine
Leucovorin
Methotrexate

Figure

  • Fig. 1 Event-free survival (EFS) of 6 patients with PCNS. Estimated 5-yr EFS rate is 83.3%.

  • Fig. 2 Patient 2 and patient 6 had their primary tumor in multiples site including cerebellum and third ventricle, and left parietal area, respectively. After systemic and intrathecal chemotherapy, their tumors were completely resolved.


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